Abstract

Hereditary medullary thyroid cancer is an aggressive cancer for which there is no standard effective systemic therapy, but which can be prevented through genetic screening and prophylactic thyroidectomy. Although this cancer accounts for roughly 17% of all pediatric thyroid cancers, a significant percentage of affected families do not "accept" screening, while many gene carriers delay or refuse prophylactic thyroid surgery for their children. Current genetic screening practices in medullary thyroid cancer are inadequate; more than 50% of index patients with hereditary medullary thyroid cancer present with a thyroid mass; up to 75% have distant metastasis. These proposed pediatric ethics guidelines focus on two ethical issues that affect at-risk children: (1) how do we identify at-risk children whose RET-positive relative refuses to disclose that they carry the mutation? (2) How do we protect RET-positive children whose parents refuse prophylactic thyroidectomy?

Document Type

Response or Comment

Publication Date

2-6-2011

Notes/Citation Information

Published in International Journal of Pediatric Endocrinology, v. 2011, 847603.

© 2011 Rosenthal and Diekema.

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Digital Object Identifier (DOI)

http://dx.doi.org/10.1155/2011/847603

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